Detection of individual sensitization patterns with different α-Gal-containing analytes provides the basis for an individual allergy diagnosis for α-Gal-sensitized patients. Higher amounts of α-Gal in pork and beef innards compared to muscle meat as indicated by a higher staining intensity are a plausible explanation for the difference in allergic symptom severity.
The patients and both hospital facilities benefitted from the interdisciplinary consultation service. The initial high costs in terms of medical staff and time was compensated by the development of diagnostic and treatment algorithms. Overall, the concept received positive feedback from patients and medical staff members.
Background
Epidermolysis bullosa (EB) is a rare genetic disorder manifesting with skin and mucosal membrane blistering in different degrees of severity.
Objective
Epidemiological data from different countries have been published, but none are available from Germany.
Methods
In this population‐based cross‐sectional study, people living with EB in Germany were identified using the following sources: academic hospitals, diagnostic laboratories and patient organization.
Results
Our study indicates an overall EB incidence of 45 per million live births in Germany. With 14.23 per million live births for junctional EB, the incidence is higher than in other countries, possibly reflecting the availability of early molecular genetic diagnostics in severely affected neonates. Dystrophic EB was assessed at 15.58 cases per million live births. The relatively low incidence found for EB simplex, 14.93 per million live births, could be explained by late or missed diagnosis, but also by 33% of cases remaining not otherwise specified. Using log‐linear models, we estimated a prevalence of 54 per million for all EB types, 2.44 for junctional EB, 12.16 for dystrophic EB and 28.44 per million for EB simplex. These figures are comparable to previously reported data from other countries.
Conclusions
Altogether, there are at least 2000 patients with EB in the German population. These results should support national policies and pharmaceutical companies in decision‐making, allow more precise planning of drug development and clinical trials, and aid patient advocacy groups in their effort to improve quality of life of people with this orphan disease.
Scabies is a globally prevalent parasitic skin infestation characterized by severe pruritus and a heterogeneous clinical picture depending on the immune status of the individual. Epidemiologic studies reveal a higher susceptibility for young, old and, in general, immunocompromised individuals. Disease-related morbidity and secondary complications due to a disrupted epidermal barrier, e.g. subsequent bacterial infections and chronic kidney disease, further add to the patients' burden. 1 With current treatment options, induction of remission is achieved in most of the patients. Specifically, the oral administration of ivermectin has substantially decreased the prevalence of scabies in communities and has proven to be effective and tolerable. 2,3 However, relapses in larger families are frequent and ivermectin is not approved for children <15 kg. Hence, evaluation of the safety of ivermectin use in infants is highly warranted. Recently, a multicentric observational study has reported on the safety and efficacy of ivermectin use in infants weighing <15 kg 4. 85% of 170 patients achieved remission and in 4% of the patients, mild adverse effects were observed. However, safety of ivermectin remains controversial, 5,6 and controlled studies in infants are scant. 7,8 We therefore retrospectively analysed 30 infants <15 kg infected with scabies. The patients were treated off-label with oral ivermectin under controlled and standardized conditions during hospitalization between 01/2018 and 01/2020. The focus of our observation was on safety and efficacy. This study was approved by the local ethics committee. After informed consent by the legal guardian(s), 30 patients [age (months), mean AE SD: 12 AE 9.2; weight (kg), mean AE SD: 8.6 AE 2.5, 16 males, 14 females] were treated with oral ivermectin after clinical and dermatoscopic diagnosis of scabies. All patients had been unsuccessfully treated Funding sources The study was supported by the Excellence Cluster 'Precision Medicine in Chronic Inflammation' (EXC 2167/1) and the Research Training Group 'Modulation of Autoimmunity' (GRK 1727/2) both from the Deutsche Forschungsgemeinschaft.
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